The first 60 days: Goblet cell adenocarcinoma of the appendix
Goblet cell adenocarcinoma is a rare appendix cancer whose cells mix mucus-filled goblet cells with neuroendocrine features, once called goblet cell carcinoid but now classed and treated as an adenocarcinoma. It is removed by right hemicolectomy, given bowel-cancer chemotherapy when it is high grade or has spread, and not treated with the somatostatin drugs used for true neuroendocrine tumours. Below, week by week, is what OnCo's record of Goblet cell adenocarcinoma of the appendix says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Expert pathology review with WHO 2019 grading; CT of chest, abdomen and pelvis; colonoscopy; tumour markers; no somatostatin receptor imaging is needed.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Diagnosis.
- RadiologistNamed in the standard of care for: Diagnosis.
- SurgeonNamed in the standard of care for: Localised disease, Peritoneal metastases.
- Medical oncologistNamed in the standard of care for: Localised disease, Peritoneal metastases, Metastatic disease.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Right hemicolectomy with lymphadenectomy for all grades; oophorectomy considered in postmenopausal women; adjuvant FOLFOX or CAPOX for node-positive or grade 2 to 3 disease.
Cytoreductive surgery with HIPEC in fit patients with limited disease, with perioperative systemic chemotherapy.
FOLFOX or CAPOX, then FOLFIRI, as for colorectal adenocarcinoma; somatostatin analogues and platinum-etoposide are not used.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example WHO 2019 grade, TNM stage after right hemicolectomy, Synaptophysin and chromograninwith mucin stains, Somatostatin receptor status, CEA, CA 19-9 and CA-125 for surveillance), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Grade 1 goblet cell adenocarcinoma, Grade 2 goblet cell adenocarcinoma, Grade 3 goblet cell adenocarcinoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis
- For my situation (diagnosis), which of the standard options do you recommend and why?Guideline options include: Expert pathology review with WHO 2019 grading; CT of chest, abdomen and pelvis; colonoscopy; tumour markers; no somatostatin receptor imaging is needed.
Localised disease
- For my situation (localised disease), which of the standard options do you recommend and why?Guideline options include: Right hemicolectomy with lymphadenectomy for all grades; oophorectomy considered in postmenopausal women; adjuvant FOLFOX or CAPOX for node-positive or grade 2 to 3 disease.
- Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), CAPOX (capecitabine, oxaliplatin), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Peritoneal metastases
- For my situation (peritoneal metastases), which of the standard options do you recommend and why?Guideline options include: Cytoreductive surgery with HIPEC in fit patients with limited disease, with perioperative systemic chemotherapy.
- Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Metastatic disease
- For my situation (metastatic disease), which of the standard options do you recommend and why?Guideline options include: FOLFOX or CAPOX, then FOLFIRI, as for colorectal adenocarcinoma; somatostatin analogues and platinum-etoposide are not used.
- Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), CAPOX (capecitabine, oxaliplatin), FOLFIRI (5-FU, leucovorin, irinotecan), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of FOLFOX (5-FU, leucovorin, oxaliplatin), HIPEC / PIPAC (intraperitoneal chemotherapy)?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “No prospective trial has ever been conducted; all treatment is extrapolated”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “The benefit of adjuvant chemotherapy in grade 1 node-negative disease is unknown”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Goblet cell adenocarcinoma of the appendix: the full pageGoblet cell adenocarcinoma is a rare appendix cancer whose cells mix mucus-filled goblet cells with neuroendocrine features, once called goblet cell carcinoid but now classed and treated as an adenocarcinoma. It is removed by right hemicolectomy, given bowel-cancer chemotherapy when it is high grade or has spread, and not treated with the somatostatin drugs used for true neuroendocrine tumours.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- HIPEC (hyperthermic intraperitoneal chemotherapy): After surgeons remove all visible tumour from the abdominal lining, the abdomen is bathed for 60-90 minutes in heated chemotherapy to kill the microscopic cells left behind.
- Colectomy: Removing the part of the colon containing the cancer along with its blood supply and lymph nodes, then joining the ends.
- Peritoneal metastasis: Spread across the lining of the abdomen, the most common way stomach cancer recurs and the hardest to treat.
- Endoscopy (EGD, EUS, ERCP): Looking inside a hollow organ with a camera on a flexible tube, taking biopsies and sometimes treating on the spot.
- Lymphadenectomy (lymph node dissection): Surgically removing the lymph nodes that drain a tumour, both to stage the cancer and to clear any spread.
Every term links to the glossary.